Between 1985 and 1987, 31 patients with sporadic olivopontocerebellar atrophy sopca and 3 patients with familial olivopontocerebellar atrophy fopca were examined in the neurologic clinic of. Opca is present in several neurodegenerative syndromes, including inherited and noninherited forms of ataxia. Pdf olivopontocerebellar atrophy with dementia syndrome. Aan members we have changed the login procedure to improve access between and the neurology journals. Olivopontoccerebellar atrophy is listed as a rare disease by the office of rare diseases ord of the national. We present seven cases of progressive ataxia with onset in childhood along with pathological findings in three patients. Olivopontocerebellar atrophy an overview sciencedirect topics. Olivopontocerebellar atrophy with dementia syndrome. Links to pubmed are also available for selected references. This produces a certain set of symptoms and needs appropriate treatment.
Olivopontocerebellar atrophy cerebellum human diseases. Evolution of sporadic olivopontocerebellar atrophy into. Olivopontocerebellar atrophy opca defines chronic progressive hereditary usually dominant, occasionally. Opca may be associated with degeneration of other systems in multisystem atrophy msa. Mri is the imaging study of choice in patients with olivopontocerebellar atrophy opca because ct scanning does not provide adequate resolution of the pons and cerebellum. The first page of the pdf of this article appears above. Sleepdisordered breathing and risk of sudden death in multiple system atrophy. Olivopontocerebellar atrophy opca is a rare condition with a prevalence estimated to be. Olivopontocerebellar atrophy free download as powerpoint presentation.
The cause of msac in people with the sporadic form is not known. Multiple system atrophy, or msa, is a rare neurological disorder that impairs your bodys involuntary functions, including. Olivopontocerebellar atrophy information page national. Full text is available as a scanned copy of the original print version. Clinical features and natural history of multiple system atrophy. Clinicopathological study of 35 cases of multiple system atrophy.
The history of multiplesystem atrophy reflects the varied clinical manifesta. Olivopontocerebellar atrophy genetic and rare diseases nih. Hereditary olivopontocerebellar atrophy opca is a specific health condition where nerves in certain part of the brain degenerate. Sleepdisordered breathing and risk of sudden death in. Olivopontocerebellar atrophy opca is a term used for a progressive condition characterized by the degeneration of nerve cells neurons in specific areas of the brain. Dsmiv denotes diagnostic and statistical manual of mental. Whatever the subtype, the term opca indicates a form of progressive ataxia distinguished by pontine flattening and cerebellar atrophy on brain. Get a printable copy pdf file of the complete article 435k, or click on a page image below to browse page by page. The authors report 23 cases of opca, eight of which were associated with msa. Olivopontocerebellar atrophy genetic and rare diseases. Mri is the imaging study of choice in patients with olivopontocerebellar atrophy opca because ct scanning does not provide. To determine the percentage of sporadic olivopontocerebellar atrophy sopca patients who later develop multiple system atrophy. Tremor, pyramidal signs and myoclonus were less common than in msa of. Pdf on dec 17, 2018, sombat muengtaweepongsa and others published.